Abstract 14210: Heart Failure Hospitalizations and Mortality Outcomes in PV142I Transthyretin Cardiomyopathy Patients Treated With Tafamidis

Abstract

Introduction: TTR amyloid cardiomyopathy (ATTR-CM) is an increasingly recognized cause of heart failure in the geriatric population. The pV142I (V122I) transthyretin gene mutation is carried in 3.4% of African Americans. It is the most common cause of hereditary ATTR-CM (hATTR-CM) in the United States with a median survival of 2.5 years from diagnosis. Tafamidis is a selective transthyretin stabilizer approved in 2019 that has been shown to improve survival in wild type and hereditary ATTR-CM with New York Heart Association class I to III symptoms. The impact of tafamidis treatment on heart failure hospitalization (HFH) and mortality outcomes in pV142I hATTR-CM requires further investigation. Hypothesis: Tafamidis decreases HFH and mortality in pV142I ATTR-CM. Methods: We conducted a retrospective chart review of patients diagnosed with ATTR-CM with pV142I mutations enrolled in Emory University's Cardiac Amyloidosis Clinic between January 2014 and February 2022. Kaplan-Meier (KM) curves stratified by tafamidis use were generated for mortality and first HFH within 2 years of initial diagnosis. Log-rank test was used to compare KM curves. A p-value < 0.05 was considered significant. Results: 111 patients in our study have pV142I hATTR-CM, including 47 (42%) prescribed tafamidis and 64 (58%) who were not prescribed. The median survival was 616 [interquartile range 277-731] days for patients treated with tafamidis and 595 [216, 731] days for untreated patients. The median time to HFH with tafamidis was 464 [167, 685] days and 233 [41, 613] days for untreated patients. Patients with tafamidis use had both lower rate of mortality (p = 0.005) and HFH (p = 0.035) than those who did not. Conclusion: In patients with pV142I hATTR-CM within our cohort, tafamidis use was associated with lower rates of mortality and heart failure hospitalization. These findings are consistent with previously described benefits in a generalized ATTR-CM population.